Congenital and Miscellaneous Abnormalities

  • Jason E. Lang
  • Robert H. Cleveland
  • Kara Palm
  • Neil Mardis
  • Edward Y. Lee
  • Umakanth Khatwa


Bronchobiliary fistula (BBF) is a rare condition characterized by communication between the tracheobronchial and biliary ductal systems. When BBF occurs, it typically is associated with trauma, cancer, or liver infection, although congenital BBF has been described. Cast, or plastic, bronchitis is a potentially fatal disorder seen relatively rarely in children. It derives its name from mucoid material which forms within the tracheobronchial tree in the form of a branching tubular cast of the airway. It characteristically has a rubbery consistency. These may be expectorated piecemeal or removed at bronchoscopy as extensive casts of the airway. Congenital diaphragmatic hernia (CDH) describes an inborn defect in the diaphragm that allows protrusion of abdominal fat and/or viscera through the opening into the thoracic cavity. The cause of this defect is not fully understood and likely is multifactorial. Patients with diaphragmatic hernias suffer from severe, often lethal, pulmonary hypoplasia. In addition to the startlingly high mortality despite medical advances, short- and long-term morbidity is significant. Primary hepatopulmonary fusion is an extremely rare condition in patients with a right-sided CDH, in which there is a fusion between the right lung and the herniated liver parenchyma through a defect in the diaphragm. There may be varying degrees of pulmonary hypoplasia associated with this condition.

In horseshoe lung, the posteroinferior bases of the right and left lungs are fused by a narrow band of normal lung tissue referred to as an “isthmus” without an intervening pleural fissure. The isthmic portion of the horseshoe lung originates from the hypoplastic sides of lung; it is located posterior to the heart, but anterior to the aorta and to the esophagus. The blood supply for the isthmus usually originates from the right pulmonary artery and is aerated by a bronchial extension from the right bronchus.


Diaphragmatic Hernia Congenital Diaphragmatic Hernia Congenital Diaphragmatic Hernia Pulmonary Hypoplasia Diaphragmatic Defect 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Tommasoni N, Gamba PG, Midrio P, Guglielmi M. Congenital tracheobiliary fistula. Pediatr Pulmonol. 2000;30(2):149–52.PubMedCrossRefGoogle Scholar
  2. 2.
    Neuhauser E, Elkin M, Landing B. Congenital direct ­communication between biliary system and respiratory tract. AMA Am J Dis Child. 1952;83(5):654–9.PubMedGoogle Scholar
  3. 3.
    de Carvalho CR, Barbas CS, Guarnieri RM, et al. Congenital bronchobiliary fistula: first case in an adult. Thorax. 1988;43(10):792–3.PubMedCrossRefGoogle Scholar
  4. 4.
    Gunlemez A, Tugay M, Elemen L, et al. Surgical experience in a baby with congenital broncho-biliary fistula. Ann Thorac Surg. 2009;87(1):318–20.PubMedCrossRefGoogle Scholar
  5. 5.
    Tekant GA, Joseph VT, Cheah SL. Congenital tracheobiliary fistula. J Pediatr Surg. 1994;29(5):594–5.PubMedCrossRefGoogle Scholar
  6. 6.
    Egrari S, Krishnamoorthy M, Yee CA, et al. Congenital bronchobiliary fistula: diagnosis and postoperative surveillance with HIDA scan. J Pediatr Surg. 1996;31(6):785–6.PubMedCrossRefGoogle Scholar
  7. 7.
    Kalayoglu M, Olcay I. Congenital bronchobiliary fistula associated with esophageal atresia and tracheo-esophageal fistula. J Pediatr Surg. 1976;11(3):463–4.PubMedCrossRefGoogle Scholar
  8. 8.
    Bringas Bollada M, Cabezas Martin MH, Martinez Sagasti F, et al. Congenital bronchobiliary fistula diagnosed in adult age. Med Intensiva. 2006;30(9):475–6.PubMedCrossRefGoogle Scholar
  9. 9.
    Peacock TB. Case in which hydatids were expectorated and one of suppuration of a hydatid cyst of the liver ­communicating with the lungs. Edinburgh Med Surg J. 1850;74:33–46.Google Scholar
  10. 10.
    Eryigit H, Urek S, Olgac G, et al. Management of acquired bronchobiliary fistula: 3 case reports and a literature review. J Cardiothorac Surg. 2007;2:52.PubMedCrossRefGoogle Scholar
  11. 11.
    Gandhi N, Kent T, Kaban JM, et al. Bronchobiliary fistula after penetrating thoracoabdominal trauma: case report and literature review. J Trauma. 2009;67(5):E143–5.PubMedCrossRefGoogle Scholar
  12. 12.
    Mitra S, Bhatia N, Dey N, Dalal U. Bronchobiliary fistula: an anesthetic challenge! J Clin Anesth. 2009;21(5):360–2.PubMedCrossRefGoogle Scholar
  13. 13.
    Annovazzi A, Viceconte G, Romano L, et al. Detection of a suspected bronchobiliary fistula by hepatobiliary scintigraphy. Ann Nucl Med. 2008;22(7):641–3.PubMedCrossRefGoogle Scholar
  14. 14.
    Flemma RJ, Anlyan WG. Tuberculous bronchobiliary fistula. Report of an unusual case with demonstration of the fistulous tract by percutaneous transhepatic cholangiography. J Thorac Cardiovasc Surg. 1965;49:198–201.PubMedGoogle Scholar
  15. 15.
    Borrie J, Shaw JH. Hepatobronchial fistula caused by hydatid disease. The Dunedin experience 1952–79. Thorax. 1981;36(1):25–8.PubMedCrossRefGoogle Scholar
  16. 16.
    Singh B, Moodley J, Sheik-Gafoor MH, et al. Conservative management of thoracobiliary fistula. Ann Thorac Surg. 2002;73(4):1088–91.PubMedCrossRefGoogle Scholar
  17. 17.
    Oettl C, Schima W, Metz-Schimmerl S, et al. Bronchobiliary fistula after hemihepatectomy: cholangiopancreaticography, computed tomography and magnetic resonance cholangiography findings. Eur J Radiol. 1999;32(3):211–5.PubMedCrossRefGoogle Scholar
  18. 18.
    Sheik-Gafoor MH, Singh B, Moodley J. Traumatic thoracobiliary fistula: report of a case successfully managed conservatively, with an overview of current diagnostic and therapeutic options. J Trauma. 1998;45(4):819–21.PubMedCrossRefGoogle Scholar
  19. 19.
    Chua HK, Allen MS, Deschamps C, et al. Bronchobiliary fistula: principles ofGoogle Scholar
  20. 20.
    Kruger J, Shpringer C, Picard E, Kerem E. Thoracic air leakage in the presentation of cast bronchitis. Chest. 2009;136:615–7.PubMedCrossRefGoogle Scholar
  21. 21.
    Seear M, Hui H, Magee F, Bohn D, Cutz E. Bronchial cast in children: a proposed classification based on nine cases and a review of the literature. Am J Respir Crit Care Med. 1997;155:364–70.PubMedGoogle Scholar
  22. 22.
    Bowen A’D, Oudjhane K, Odagiri K, Liston SL, Cumming WA, Oh KS. Plastic bronchitis: large, branching, mucoid bronchial casts in children. Am J Roentgenol. 1985;144:371–5.Google Scholar
  23. 23.
    Brogan TV, Finn LS, Pyskaty DJ, Reddding GJ, Ricker D, Inglis A, et al. Plastic bronchitis in children: a case series and review of the medical literature. Pediatr Pulmonol. 2002;34:482–7.PubMedCrossRefGoogle Scholar
  24. 24.
    Schultz KD, Oermann CM. Treatment of cast bronchitis with low-dose oral azithromycin. Pediatr Pulmonol. 2003;35:139–43.PubMedCrossRefGoogle Scholar
  25. 25.
    Costello JM, Steinhorn D, McColley S, Gerber ME, Kumar SP. Treatment of plastic bronchitis in a Fontan patient with tissue plasminogen activator: a case report and review of the literature. Pediatrics. 2002;109:e67.PubMedCrossRefGoogle Scholar
  26. 26.
    Wenstrom KD, Weiner CP, Hanson JW. A five-year statewide experience with congenital diaphragmatic hernia. Am J Obstet Gynecol. 1991;165:838–42.PubMedGoogle Scholar
  27. 27.
    Langham Jr MR, Kays DW, Ledbetter DJ, Frentzen B, Sanford LL, Richards DS. Congenital diaphragmatic hernia: epidemiology and outcome. Clin Perinatol. 1996;23:671–88.PubMedGoogle Scholar
  28. 28.
    Stege G, Fenton A, Jaffray B. Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia. Pediatrics. 2003;112:532–35.PubMedCrossRefGoogle Scholar
  29. 29.
    Sweed Y, Puri P. Congenital diaphragmatic hernia: influence of associated malformations on survival. Arch Dis Child. 1993;69:68–70.PubMedCrossRefGoogle Scholar
  30. 30.
    Holder AM, Klaassens M, Tibboel D, de Klein A, Lee B, Scott DA. Genetic factors in congenital diaphragmatic hernia. Am J Hum Genet. 2007;80:825–45.PubMedCrossRefGoogle Scholar
  31. 31.
    Moore KL, Persaud TVN. The developing human: clinically oriented anatomy. 8th ed. Philadelphia: Saunders; 2008.Google Scholar
  32. 32.
    Babiuk RP, Zhang W, Clugston R, Allan DW, Greer JJ. Embryological origins and development of the rat diaphragm. J Comp Neurol. 2003;455:477–87.PubMedCrossRefGoogle Scholar
  33. 33.
    Clugston RD, Klattig J, Englert C, Clagett-Dame M, Martinovic J, Benachi A, et al. Teratogen-induced, dietary and genetic models of congenital diaphragmatic hernia share a common mechanism of pathogenesis. Am J Pathol. 2006;169:1541–49.PubMedCrossRefGoogle Scholar
  34. 34.
    Montedonico S, Nakazawa N, Puri P. Congenital diaphragmatic hernia and retinoids: searching for an etiology. Pediatr Surg Int. 2008;24:755–61.PubMedCrossRefGoogle Scholar
  35. 35.
    Mortell A, Montedonico S, Puri P. Animal models in pediatric surgery. Pediatr Surg Int. 2006;22:111–28.PubMedCrossRefGoogle Scholar
  36. 36.
    Baglaj SM, Czernik J. Nitrofen-induced congenital diaphragmatic hernia in rat embryo: what model? J Pediatr Surg. 2004;39:24–30.PubMedCrossRefGoogle Scholar
  37. 37.
    Noble BR, Babiuk RP, Clugston RD, Underhill TM, Sun H, Kawaguchi R. Mechanisms of action of the congenital diaphragmatic hernia-inducing teratogen nitrofen. Am J Physiol Lung Cell Mol Physiol. 2007;293:L1079–87.PubMedCrossRefGoogle Scholar
  38. 38.
    Major D, Cadenas M, Fournier L, Leclerc S, Lefebvre M, Cloutier R. Retinol status of newborn infants with congenital diaphragmatic hernia. Pediatr Surg Int. 1998;13:547–49.PubMedCrossRefGoogle Scholar
  39. 39.
    Keijer R, Liu J, Deimling J, Tibboel D, Post M. Dual-hit hypothesis explains pulmonary hypoplasia in the nitrofen model of congenital diaphragmatic hernia. Am J Pathol. 2000;156:1299–306.CrossRefGoogle Scholar
  40. 40.
    The Congenital Diaphragmatic Hernia Study Group. Defect size determines survival in infants with congenital diaphragmatic hernia. Pediatrics. 2007;120:e651–57.CrossRefGoogle Scholar
  41. 41.
    Torfs CP, Curry CJ, Bateson TF, Honore LH. A population-based study of congenital diaphragmatic hernia. Teratology. 1992;46:555–65.PubMedCrossRefGoogle Scholar
  42. 42.
    Al-Salem AH, Nawaz A, Matta H, Jacobsz A. Herniation through the foramen of Morgagni: early diagnosis and treatment. Pediatr Surg Int. 2002;18:93–7.PubMedCrossRefGoogle Scholar
  43. 43.
    Cantrell JR, Haller JA, Ravitch MM. A syndrome of ­congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet. 1958;107:602–14.PubMedGoogle Scholar
  44. 44.
    Jeanty C, Nien JK, Espinoza J, Kusanovic JP, Goncalves LF, Qureshi F, et al. Pleural and pericardial effusion: a potential ultrasonographic marker for the prenatal differential diagnosis between congenital diaphragmatic eventration and congenital diaphragmatic hernia. Ultrasound Obstet Gynecol. 2007;29:378–87.PubMedCrossRefGoogle Scholar
  45. 45.
    Metkus AP, Filly RA, Stringer MD, Harrison MR, Adzick NS. Sonographic predictors of survival in fetal diaphragmatic hernia. J Pediatr Surg. 1996;31:148–51.PubMedCrossRefGoogle Scholar
  46. 46.
    Lipshutz GS, Albanese CT, Feldstein VA, Jennings RW, Housley HT, Beech R, et al. Prospective analysis of ­lung-to-head ratio predicts survival for patients with prenatally diagnosed congenital diaphragmatic hernia. J Pediatr Surg. 1997;32:1634–36.PubMedCrossRefGoogle Scholar
  47. 47.
    Jani J, Keller RL, Benachi A, Nicolaides KH, Favre R, Gratacos E. Prenatal prediction of survival in isolated left-sided diaphragmatic hernia. Ultrasound Obstet Gynecol. 2006;27:18–22.PubMedCrossRefGoogle Scholar
  48. 48.
    Laudy JA, Van Gucht M, Van Dooren MF, Wladimiroff JW, Tibboel D. Congenital diaphragmatic hernia: an evaluation of the prognostic value of the lung-to-head ratio and other prenatal parameters. Prenatal Diagn. 2003;23:634–39.CrossRefGoogle Scholar
  49. 49.
    Sbragia L, Paek BW, Filly RA, Harrison MR, Farrell JA, Farmer DL, et al. Congenital diaphragmatic hernia without herniation of the liver: does the lung-to-head ratio predict survival? J Ultrasound Med. 2000;19:845–48.PubMedGoogle Scholar
  50. 50.
    Heling KS, Wauer RR, Hammer H, Bollman R, Chaoui R. Reliability of the lung-to-head ratio in predicting outcome and neonatal ventilation parameters in fetuses with congenital diaphragmatic hernia. Ultrasound Obstet Gynecol. 2005;25:112–18.PubMedCrossRefGoogle Scholar
  51. 51.
    Arkovitz MS, Russo M, Devine P, Budhorick N, Stolar CJ. Fetal lung-head ratio is not related to outcome for antenatal diagnosed congenital diaphragmatic hernia. J Pediatr Surg. 2007;42:107–10.PubMedCrossRefGoogle Scholar
  52. 52.
    Bahmaie A, Hughes SW, Clark T, Milner A, Saunders J, Tilling K, et al. Serial fetal lung volume measurements using three-dimensional ultrasound. Ultrasound Obstet Gynecol. 2000;16:154–58.PubMedCrossRefGoogle Scholar
  53. 53.
    Ruano R, Joubin L, Sonigo P, Benachi A, Aubry MC, Thalabard JC, et al. Fetal lung volume estimated by 3-dimensional ultrasonography and magnetic resonance imaging in cases with isolated congenital diaphragmatic hernia. J Ultrasound Med. 2004;23:353–58.PubMedGoogle Scholar
  54. 54.
    Jani JC, Cannie M, Peralta CF, Deprest JA, Nicolaides KH, Dymarkowski S. Lung volumes in fetuses with congenital diaphragmatic hernia: comparison of 3D US and MR imaging assessments. Radiology. 2007;244:575–82.PubMedCrossRefGoogle Scholar
  55. 55.
    Büsing KA, Kilian AK, Schaible T, Endler C, Schaffelder R, Neff KW. MR relative fetal lung volume in congenital diaphragmatic hernia: survival and need for extracorporeal membrane oxygenation. Radiology. 2008;248:240–46.PubMedCrossRefGoogle Scholar
  56. 56.
    Barnewolt CE, Kunisaki SM, Fauza DO, Nemes LP, Estroff JA, Jennings RW. Percent predicted lung volumes as measured on fetal magnetic resonance imaging: a useful biometric parameter for risk stratification in congenital diaphragmatic hernia. J Pediatr Surg. 2007;42:193–7.PubMedCrossRefGoogle Scholar
  57. 57.
    Büsing KA, Kilian AK, Schaible T, Dinter DJ, Neff KW. MR lung volume in fetal congenital diaphragmatic hernia: logistic regression analysis – mortality and extracorporeal membrane oxygenation. Radiology. 2008;248:233–39.PubMedCrossRefGoogle Scholar
  58. 58.
    Kunisaki SM, Barnewolt CE, Estroff JA, Nemes LP, Jennings RW, Wilson JM, et al. Liver position is a prenatal factor of prosthetic repair in congenital diaphragmatic hernia. Fetal Diagn Ther. 2008;23:258–62.PubMedCrossRefGoogle Scholar
  59. 59.
    Harrison MR, Adzick NS, Bullard KM, Farrell JA, Howell LJ, Rosen MA, et al. Correction of congenital diaphragmatic hernia in utero VII: a prospective trial. J Pediatr Surg. 1997;32:1637–42.PubMedCrossRefGoogle Scholar
  60. 60.
    Harrison MR, Keller RL, Hawgood SB, Kitterman JA, Sandberg PL, Farmer DL, et al. A Randomized trial of fetal endoscopic tracheal occlusion for severe fetal congenital diaphragmatic hernia. N Engl J Med. 2003;349:1916–24.PubMedCrossRefGoogle Scholar
  61. 61.
    Lally KP, Bagolan P, Hosie S, Lally PA, Stewart M, Cotton CM, et al. Corticosteroids for fetuses with congenital diaphragmatic hernia: can we show benefit? J Pediatr Surg. 2006;41:668–74.PubMedCrossRefGoogle Scholar
  62. 62.
    Montedonico S, Sugimoto K, Felle P, Bannigan J, Puri P. Prenatal treatment with retinoic acid promotes pulmonary alveologenesis in the nitrofen model of congenital diaphragmatic hernia. J Pediatr Surg. 2008;43:500–7.PubMedCrossRefGoogle Scholar
  63. 63.
    Nakayama DK, Motoyama EK, Mutich RL, Koumbourlis AC. Pulmonary function in newborns after repair of congenital diaphragmatic hernia. Pediatr Pulmonol. 1991;11:49–55.PubMedCrossRefGoogle Scholar
  64. 64.
    Stevens TP, Chess PR, McConnochie KM, Sinkin RA, Guillet R, Maniscalco WM, et al. Survival in early- and late-term infants with congenital diaphragmatic hernia treated with extracorporeal membrane oxygenation. Pediatrics. 2002;110:590–96.PubMedCrossRefGoogle Scholar
  65. 65.
    Koumbourlis AC, Wung JT, Stolar CJ. Lung function in infants after repair of congenital diaphragmatic hernia. J Pediatr Surg. 2006;41:1716–21.PubMedCrossRefGoogle Scholar
  66. 66.
    Dotta A, Palamides S, Braguglia A, Crescenzi F, Ronchetti MP, Calzolari F, et al. Lung volumes and distribution of ventilation in survivors to congenital diaphragmatic hernia (CDH) during infancy. Pediatr Pulmonol. 2007;42:600–4.PubMedCrossRefGoogle Scholar
  67. 67.
    Jaillard SM, Pierrat V, Dubois A, Truffert P, Lequien P, Wurtz AJ, et al. Outcome at 2 years of infants with congenital diaphragmatic hernia: a population-based study. Ann Thorac Surg. 2003;75:250–56.PubMedCrossRefGoogle Scholar
  68. 68.
    Peetsold MG, Vonk-Noordegraaf A, Heij HH, Gemke R. Pulmonary function and exercise testing in adult survivors of congenital diaphragmatic hernia. Pediatr Pulmonol. 2007;42:325–31.PubMedCrossRefGoogle Scholar
  69. 69.
    Vanamo K, Rintala R, Sovijärvi A, Jääskeläinen J, Turpeinen M, Lindahl H, et al. Long-term pulmonary sequelae in survivors of congenital diaphragmatic defects. J Pediatr Surg. 1996;31:1096–100.PubMedCrossRefGoogle Scholar
  70. 70.
    Greenwood RD, Rosenthal A, Nadas AS. Cardiovascular abnormalities associated with congenital diaphragmatic hernia. Pediatrics. 1976;57:92–7.PubMedGoogle Scholar
  71. 71.
    Cuniff C, Jones KL, Jones MC. Patterns of malformation in children with congenital diaphragmatic defects. J Pediatr. 1990;116:258–61.CrossRefGoogle Scholar
  72. 72.
    Fauza DO, Wilson JM. Congenital diaphragmatic hernia and associated anomalies: their incidence, identification, and impact on prognosis. J Pediatr Surg. 1994;29:1113–7.PubMedCrossRefGoogle Scholar
  73. 73.
    Martínez-Frías ML, Prieto L, Urioste M, Bermejo E. Clinical/epidemiological analysis of congenital anomalies associated with diaphragmatic hernia. Am J Med Genet. 1996;62:71–6.PubMedCrossRefGoogle Scholar
  74. 74.
    Migliazza L, Otten C, Xia H, Rodriguez JI, Diez-Pardo JA, Tovar JA. Cardiovascular malformations in congenital diaphragmatic hernia: human and experimental studies. J Pediatr Surg. 1999;34:1352–8.PubMedCrossRefGoogle Scholar
  75. 75.
    Cohen MS, Rychik J, Bush DM, Tian ZY, Howell LJ, Adzick NS, et al. Influence of congenital heart disease on survival in children with congenital diaphragmatic hernia. J Pediatr. 2002;141:25–30.PubMedCrossRefGoogle Scholar
  76. 76.
    Graziano JN. Cardiac anomalies in patients with congenital diaphragmatic heria and their prognosis: a report from the congenital diaphragmatic hernia study group. J Pediatr Surg. 2005;40:1045–50.PubMedCrossRefGoogle Scholar
  77. 77.
    Vanamo K, Rintala RJ, Lindahl H, Louhimo I. Long-term gastrointestinal morbidity in patients with congenital diaphragmatic defects. J Pediatr Surg. 1996;31:551–54.PubMedCrossRefGoogle Scholar
  78. 78.
    Koot VC, Bergmeijer JH, Bos AP, Molenaar JC. Incidence and management of gastroesophageal reflux after repair of congenital diaphragmatic hernia. J Pediatr Surg. 1993;28:48–52.PubMedCrossRefGoogle Scholar
  79. 79.
    Kieffer J, Sapin E, Berg A, Beaudoin S, Bargy F, Helardot PG. Gastroesophageal reflux after repair of congenital diaphragmatic hernia. J Pediatr Surg. 1995;30:1330–3.PubMedCrossRefGoogle Scholar
  80. 80.
    Su W, Berry M, Puligandla PS, Aspirot A, Flageole H, Laberge JM. Predictors of gastroesophageal reflux in neonates with congenital diaphragmatic hernia. J Pediatr Surg. 2007;42:1639–43.PubMedCrossRefGoogle Scholar
  81. 81.
    van Dooren MF, Goemaere N, de Klein A, Tibboel D, de Krijger RR. Postmortem findings and clinicopathological correlation in congenital diaphragmatic hernia. Pediatr Dev Pathol. 2004;7:459–67.PubMedCrossRefGoogle Scholar
  82. 82.
    Benjamin DR, Juul S, Siebert JR. Congenital posterolateral diaphragmatic hernia: associated malformations. J Pediatr Surg. 1988;23:899–903.PubMedCrossRefGoogle Scholar
  83. 83.
    Vanamo K, Peltonen J, Rintala R, Lindahl H, Jääskeläinen J, Louhimo I. Chest wall and spinal deformities in adults with congenital diaphragmatic defects. J Pediatr Surg. 1996;31:851–54.PubMedCrossRefGoogle Scholar
  84. 84.
    Lund DP, Mitchell J, Kharasch V, Quigley S, Kuehn M, Wilson JM. Congenital diaphragmatic hernia: the hidden morbidity. J Pediatr Surg. 1994;29:258–62.PubMedCrossRefGoogle Scholar
  85. 85.
    Arena F, Romeo C, Calabrò MP, Antonuccio P, Arena S, Romeo G. Long-term functional evaluation of diaphragmatic motility after repair of congenital diaphragmatic hernia. J Pediatr Surg. 2005;40:1078–81.PubMedCrossRefGoogle Scholar
  86. 86.
    Stolar C, Crisafi MA, Driscoll YT. Neurocognitive outcome for neonates treated with extracorporeal membrane oxygenation: are infants with congenital diaphragmatic hernia different? J Pediatr Surg. 1995;30:366–71.PubMedCrossRefGoogle Scholar
  87. 87.
    Stoll C, Alembik Y, Dott B, Roth MP. Associated malformations in cases with congenital diaphragmatic hernia. Genet Couns. 2008;19:331–9.PubMedGoogle Scholar
  88. 88.
    Tibboel D, Gaag A. Etiologic and genetic factors in congenital diaphragmatic hernia. Clin Perinatol. 1996;23:689–99.PubMedGoogle Scholar
  89. 89.
    Cigdem MK, Onen A, Okur H, Otcu S. Associated malformations in Morgagni hernia. Pediatr Surg Int. 2007;23:1101–3.PubMedCrossRefGoogle Scholar
  90. 90.
    Stolar CJ, Dillon PW. Pediatric surgery. 6th ed. Philadelphia: Mosby; 2006.Google Scholar
  91. 91.
    Tanaka S, Kubota M, Yagi M, et al. Treatment of a case with right-sided diaphragmatic hernia associated with an abnormal vessel communication between a herniated liver and the right lung. J Pediatr Surg. 2006;41(3):E25–8.PubMedCrossRefGoogle Scholar
  92. 92.
    Robertson DJ, Harmon CM, Goldberg S. Right congenital diaphragmatic hernia associated with fusion of the liver and the lung. J Pediatr Surg. 2006;41(6):E9–10.PubMedCrossRefGoogle Scholar
  93. 93.
    Slovis TL, Farmer DL, Berdon WE, et al. Hepatic pulmonary fusion in neonates. Am J Radiol. 2000;174:229–33.Google Scholar
  94. 94.
    Kluth D, Tenbrinck R, Ekesparre M, et al. The natural history of congenital diaphragmatic hernia and pulmonary hypoplasia in embryo. J Pediatr Surg. 1993;28:456–63.PubMedCrossRefGoogle Scholar
  95. 95.
    Katz S, Kidron D, Litmanovitz I, et al. Fibrous fusion between the liver and the lung: an unusual complication of right congenital diaphragmatic hernia. J Pediatr Surg. 1998;33(5):766–7.PubMedCrossRefGoogle Scholar
  96. 96.
    Van MK, Lou SB. Congenital diaphragmatic hernia: the neonatologist’s perspective. Pediatr Rev. 1999;20:e79–87.CrossRefGoogle Scholar
  97. 97.
    Keller RL, Aaroz PA, Hawgoog S, et al. MR imaging of hepato pulmonary fusion in neonates. Am J Radiol. 2003;180:438–40.Google Scholar
  98. 98.
    Nadroo AM, Levshina R, Tugertimur A, et al. Congenital diaphragmatic hernia: atypical presentation. J Perinat Med. 1999;27(4):276–8.PubMedCrossRefGoogle Scholar
  99. 99.
    Bohn D. Congenital diaphragmatic hernia. Am J Respir Crit Care Med. 2002;166:911–5.PubMedCrossRefGoogle Scholar
  100. 100.
    Lee EY, Siegel MJ, Hildebolt CF, Gutierrez FR, Fallah JH, Bhalla S. Evaluation of thoracic aortic anomalies in pediatric patients and young adults: comparison of axial, multiplanar, and 3D images. AJR. 2004;183:777–84.Google Scholar
  101. 101.
    Lee EY, Siegel MJ, Sierra LM, Foglia RP. Evaluation of angio architecture of pulmonary sequestration in pediatric patients using 3D MDCT angiography. AJR. 2004;183:183–8.PubMedGoogle Scholar
  102. 102.
    Lee EY, Dillon JE, Callahan MJ, Voss SD. 3D MDCT angiographic evaluation of extralobar sequestration with anomalous venous drainage into left internal mammary vein in a pediatric patient. Br J Radiol. 2006;79(945):e99–e102.PubMedCrossRefGoogle Scholar
  103. 103.
    Frank JL, Poole CA, Rosas G. Horseshoe lung: clinical, pathologic, and radiologic features and a new plain film finding. Am J Roentgenol. 1986;146:217–26.Google Scholar
  104. 104.
    Kelly D, Mroczek EC, Galliani CA, et al. Horseshoe lung and cross over segment: a unifying concept. Perspect Pediatr Pathol. 1995;18:183–213.Google Scholar
  105. 105.
    Paterson A. Imaging evaluation of congenital lung anomalies in infants and children. Radiol Clin N Am. 2005;43:303–23.PubMedCrossRefGoogle Scholar
  106. 106.
    Freedom RM, Burrows PE, Moes CA. Horseshoe lung: report of five cases. Am J Roentgenol. 1986;146:211–5.Google Scholar
  107. 107.
    Dupuis C, Remy J, Remy-Jardin M, et al. The “horseshoe” lung: six new cases. Pediatr Pulmonol. 1994;17(2):124–30.PubMedCrossRefGoogle Scholar
  108. 108.
    Hawass ND, Badwai AM, Al-Muzrakehi M, et al. Horseshoe lung: differential diagnosis. Pediatr Radiol. 1990;20:580–4.PubMedCrossRefGoogle Scholar
  109. 109.
    Chen SJ, Li YW, Wu MH, et al. Crossed ectopic left lung with fusion to the right lung: a variant of horseshoe lung? Am J Roentgenol. 1997;168:1347–8.Google Scholar
  110. 110.
    Takeda K, Kato N, Nakagawa T, et al. Horseshoe lung without respiratory distress. Pediatr Radiol. 1990;20(8):604.PubMedCrossRefGoogle Scholar
  111. 111.
    Takahashi M, Murata K, Yamori M, et al. Horseshoe lung: demonstration by electron-beam CT. Br J Radiol. 1997;70:964–6.PubMedGoogle Scholar
  112. 112.
    Lee EY, Dorkin H, Vargas SO. Congenital pulmonary ­malformations in pediatric patients: review and update on etiology, classification, and imaging findings. Radiol Clin North Am. 2011;49:921–48.PubMedCrossRefGoogle Scholar

Further Reading

  1. Figa FH, Yoo SJ, Burrows PE, et al. Horseshoe lung-A case report with unusual bronchial and pleural anomalies and a proposed new classification. Pediatr Radiol. 1993;23:44–7.PubMedCrossRefGoogle Scholar
  2. Hawass ND, Badawi MG, Fatani JAETAL. Horseshoe lung with multiple congenital anomalies. Case report and review of the literature. Acta Radiol. 1987;28(6):751–4.PubMedCrossRefGoogle Scholar
  3. Manner J, Jakob C, Steding G, et al. Horseshoe lung: report on a new variant-“inverted” horseshoe lung – with embryological reflections on the formal pathogenesis of horseshoe lungs. Ann Anat. 2001;183(3):261–5.PubMedCrossRefGoogle Scholar
  4. Takeda K, Kato N, Nakagawa T, et al. Horseshoe lung without respiratory distress. Pediatr Radiol. 1990;20:604.PubMedCrossRefGoogle Scholar
  5. Tilea B, Garel C, Delezoide A, et al. Prenatal diagnosis of horseshoe lung: contribution of MRI. Pediatr Radiol. 2005;35:1010–3.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  • Jason E. Lang
    • 1
  • Robert H. Cleveland
    • 2
    • 3
  • Kara Palm
    • 4
  • Neil Mardis
    • 5
  • Edward Y. Lee
    • 6
  • Umakanth Khatwa
    • 7
  1. 1.Department of Pulmonology, Allergy and ImmunologyNemours Children’s Clinic/Mayo Clinic College of MedicineJacksonvilleUSA
  2. 2.Department of RadiologyHarvard Medical SchoolBostonUSA
  3. 3.Departments of Radiology and Medicine, Division of Respiratory DiseasesChildren’s Hospital BostonBostonUSA
  4. 4.Division of Respiratory DiseasesChildren’s Hospital BostonBostonUSA
  5. 5.Department of RadiologyUniversity of Missouri-Kansas City School of MedicineKansas CityUSA
  6. 6.Division of Thoracic Imaging, Department of Radiology and Medicine, Pulmonary DivisionChildren’s Hospital Boston, Harvard Medical SchoolBostonUSA
  7. 7.Division of Respiratory Diseases, Department of MedicineChildren’s Hospital BostonBostonUSA

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